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Motor Neurone disease.
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease and Motor Neurone disease (MND) is a specific disease that causes the death of neurons which control voluntary muscles. Some also use the term motor neuron disease for a group of conditions of which ALS is the most common. ALS is characterized by stiff muscles, muscle twitching, and gradually worsening weakness due to muscles decreasing in size. This results in difficulty speaking, swallowing, and eventually breathing. The cause is not known in 90% to 95% of cases. About 5–10% of cases are inherited from a person's parents. About half of these genetic cases are due to one of two specific genes. The diagnosis is based on a person's signs and symptoms with testing done to rule out other potential causes. No cure for ALS is known. A medication called riluzole may extend life by about two to three months. Non-invasive ventilation may result in both improved quality and length of life. The disease usually starts around the age of 60 and in inherited cases around the age of 50. The average survival from onset to death is two to four years. About 10% survive longer than 10 years. Most die from respiratory failure. In much of the world, rates of ALS are unknown. In Europe and the United States the disease affects about two people per 100,000 per year. Descriptions of the disease date back to at least 1824 by Charles Bell. In 1869, the connection between the symptoms and the underlying neurological problems was first described by Jean-Martin Charcot, who in 1874 began using the term amyotrophic lateral sclerosis. It became well known in the United States in the 20th century when in 1939 it affected the baseball player Lou Gehrig and later worldwide when physicist Stephen Hawking, diagnosed in 1963 and expected to die within two years, became famous.In 2014, videos of the ice bucket challenge went viral on the World Wide Web and increased public awareness of the condition. Classification ALS is a motor neuron disease, also spelled "motor neurone disease", which is a group of neurological disorders that selectively affect motor neurons, the cells that control voluntary muscles of the body, including amyotrophic lateral sclerosis (ALS), primary lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy, pseudobulbar palsy, and spinal muscular atrophy. ALS itself can be classified a few different ways: by how fast the disease progresses (slow vs fast progressors), by whether it is inherited or sporadic, and by where it starts. Most commonly (~70% of the time) the limbs are affected first. In this case, neurons in the brain (upper motor neurons) and in the spinal cord (lower motor neurons) are dying and this form is called "limb onset". In about 25% of cases, muscles in the face, mouth, and throat are affected first because motor neurons in the part of the brain stem called the medulla oblongata (formerly called the "bulb") start to die first along with lower motor neurons. This form is called "bulbar onset". In about 5% of cases muscles in the trunk of the body are affected first. In all cases the disease spreads and affects other regions. The symptoms may also be limited to one spinal region. Those with leg amyotrophic diplegia and brachial amyotrophic diplegia have a longer survival compared to classic onset ALS. End-stage symptoms As; Motor Neurone disease progresses to its final phase, a person with the condition will probably experience: * increasing body paralysis, which means they'll need help with most daily activities * significant shortness of breath Eventually, non-invasive breathing assistance won't be enough to compensate for the loss of normal lung function. At this stage, most people with Motor Neurone disease become increasingly drowsy before falling into a deep sleep, where they usually die peacefully.